系统性红斑狼疮 皮肌炎和系统性硬皮病伴发脂膜炎26例临床分析

Clinical Analysis of 26 Cases with Subcutaneous Panniculitis in Syste mic Lupus Erythematosus , Dermatomyositis and Progressive Systemic Sclerosis Pat ients

目的 探讨系统性红斑狼疮 (SLE)、皮肌炎 (DM)和系统性硬皮病 (PSS)伴发脂膜炎的临床、免疫、病理学特征、发病机理及治疗。方法 对 19例SLE、6例DM和 1例PSS伴发脂膜炎者的有关资料进行分析。结果 脂膜炎发生率为 2 .7% ,其中SLE2 .6% ,DM 5.3 % ,PSS1.1%。表现为单发或多发痛性皮下结节、斑块和溃疡 ,多见于四肢(57.7% )。组织病理学改变为脂肪小叶坏死、间质血管炎、栓塞形成及炎性细胞浸润。经皮质类固醇 +免疫抑制剂等治疗 ,可有明显改善。 3例分别死于败血症和成人呼吸窘迫综合征。结论 脂膜炎为SLE、DM和PSS少见的并发症 ;以发热、痛性皮下结节和溃疡为其特征 ;病理以脂肪小叶坏死为主 ,血管炎及栓塞可能是其发病的重要因素 ;

Objective To investigate clinical manifestatio ns,immunol and pathologic characteristics,mechanism and therapy of subcutaneous panniculitis in systemic lupus erythematosus(SLE),dermatomyositis(DM) and progre ssive systemic sclerosis(PSS). Methods To retrospectively study the data of 19 SLE,6 DM and 1 PSS patients with panniculitis in detail. Results The total morbidity of panniculitis was 2.7%,incl uding 2.6% of SLE,5.3%DM,1.1% PSS,which consisted of single/multiples situate d subcutaneous nodules and ulcers with pain,most at extremities(57.7%).The histo pathologic findings showed that there were necrosis of lipid lobules,infiltratio n of inflammatory cell and lobules septal vasculitis and thrombosis.Majority of the patients responded well to treatment of corticosteroid combinated immunosupr essive.Three patients with multiple ulcers died from septicemia and adult respir atory distress syndrome (ARDS). Conclusion Punniculitis is a rare complication in SLE,DM ,and PSS,and consisted of subcutanea nodes and ulcer with pain;the main hist opathologic findings is necrosis of lipid lobules;lobules septal vasculitis and thrombosis seems to be important factors in pathogenesis of punniculitis.Majorit y of the patients respond well to corticosteroid combinated immunosuppressive.